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. 2017 Jul;23(4):e287-e293.
doi: 10.1111/hae.13275. Epub 2017 Jun 2.

The effects of joint disease, inhibitors and other complications on health-related quality of life among males with severe haemophilia A in the United States

J M Soucie  1 , S D Grosse  1 , A-E-A Siddiqi  1 , V Byams  1 , J Thierry  1 , M M Zack  2 , A Shapiro  3 , N Duncan  3 ; Hemophilia Treatment Centers Network
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The effects of joint disease, inhibitors and other complications on health-related quality of life among males with severe haemophilia A in the United States

J M Soucie et al. Haemophilia. 2017 Jul.

Abstract

Introduction: Health-related quality of life (HRQoL) is reduced among persons with haemophilia. Little is known about how HRQoL varies with complications of haemophilia such as inhibitors and joint disease. Estimates of preference-based HRQoL measures are needed to model the cost-effectiveness of prevention strategies.

Aim: We examined the characteristics of a national sample of persons with severe haemophilia A for associations with two preference-based measures of HRQoL.

Methods: We analysed utility weights converted from EuroQol 5 Dimensions (EQ-5D) and the Short Form 6 Dimensions (SF-6D) scores from 1859 males aged ≥14 years with severe haemophilia A treated at 135 US haemophilia treatment centres in 2005-2011. Bivariate and regression analyses examined age-group-specific associations of HRQoL with inhibitor status, overweight/obesity, number of bleeds, viral infections, indicators of liver and joint disease, and severe bleeding at the time of the first HRQoL measurement.

Results: Overall mean HRQoL utility weight values were 0.71 using the SF-6D and 0.78 using the EQ-5D. All studied patient characteristics except for overweight/obesity were significantly associated with HRQoL in bivariate analyses. In a multivariate analysis, only joint disease was significantly associated with utility weights from both HRQoL measures and across all age groups. After adjustment for joint disease and other variables, the presence of an inhibitor was not significantly associated with HRQoL scores from either of the standardized assessment tools.

Conclusion: Clinically significant complications of haemophilia, especially joint disease, are strongly associated with HRQoL and should be accounted for in studies of preference-based health utilities for people with haemophilia.

Keywords: haemophilia; health-related Quality of Life; inhibitors.

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Conflict of interest statement

DISCLOSURES

The authors stated that they had no interests which might be perceived as posing a conflict or bias. The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention.

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References

    1. Wight J, Paisley S. The epidemiology of inhibitors in haemophilia A: a systematic review. Haemophilia. 2003;9:418–435. - PubMed
    1. Soucie JM, Symons J, Evatt B, Brettler D, Huszti H, Linden J. Home-based factor infusion therapy and hospitalization for bleeding complications among males with haemophilia. Haemophilia. 2001;7:198–206. - PubMed
    1. Walsh CE, Soucie JM, Miller CH, United States Hemophilia Treatment Center Network Impact of inhibitors on hemophilia A mortality in the United States. Am J Hematol. 2015;90:400–405. - PubMed
    1. Guh S, Grosse SD, McAlister S, Kessler CM, Soucie JM. Healthcare expenditures for males with haemophilia and employer-sponsored insurance in the United States, 2008. Haemophilia. 2012;18:268–275. - PMC - PubMed
    1. Guh S, Grosse SD, McAlister S, Kessler CM, Soucie JM. Health care expenditures for Medicaid-covered males with haemophilia in the United States, 2008. Haemophilia. 2012;18:276–283. - PMC - PubMed

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